Pancreas
Cysts
Cystic fibrosis

Author: Deepali Jain, M.D. (see Authors page)

Revised: 11 December 2017, last major update August 2012

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: Cystic fibrosis[TI] pancreas[TI] free full text[sb]

Cite this page: Jain, D. Cystic fibrosis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreascysticfibrosis.html. Accessed May 27th, 2018.
Definition / general
  • Autosomal recessive disorder affecting most critically the lungs; also pancreas, liver, intestines; characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions (Wikipedia: Cystic Fibrosis [Accessed 11 December 2017])
  • Incidence: 1 in 20 in U.S. are carriers
  • Most common mutation is #708 (seen in 70% with disease)
  • Mutations cause reduced chloride ion in secretions, thicker respiratory secretions, upper respiratory infections
  • Complications: pancreatic insufficiency late in disease course in 90%, diabetes, malabsorption, pancreatitis
  • Mutations also cause defective cilia and infertility; may cause meconium ileus (5 - 10%), intussusception
  • Cysts form secondary to ductal obstruction due to thick, tenacious secretions, variable numbers of cysts range from 1 to 3 mm, very rarely entire pancreas replaced by multiple macroscopic cysts, termed "pancreatic cystosis"
Gross images

Images hosted on other servers:

At autopsy, pancreas
is mucoid and
slightly smaller
than normal

Microscopic (histologic) description
  • Grade I: accumulation of secretion
  • Grade II: exocrine atrophy
  • Grade III: atrophy with lipomatosis
  • Grade IV: fibrosis with total obliteration of the exocrine glands and ducts with scattered islets of Langerhans

  • Pancreatic ducts diffusely dilated and filled with numerous lamellated concretions
  • Associated with fibrosis
  • Nondiabetic patients have fibrocystic changes with normal islets, prominent nesidioblastosis, some persisting exocrine tissue
  • Young adult diabetic patients have total loss of exocrine pancreas with fat replacement, no nesidioblastosis, reduced islets (Hum Pathol 1984;15:278)
Microscopic (histologic) images

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Abnormally viscid secretions from exocrine
glands are round eosinophil masses
distending and obstructing pancreatic ducts,
leading to destruction of parenchyma and fibrosis

Positive stains
Videos

Histopathology pancreas - cystic fibrosis

Differential diagnosis
  • Kartagener syndrome: defective cilia syndrome